Surgical correction of macroglossia in Beckwith-Wiedemann syndrome

Two cases of Beckwith-Wiedemann syndrome have been reported. The children were large at birth and had an umbilical defect and macroglossia. After considering several alternatives, the elliptical (modified Butlin-Handley), U-shaped excision was used in case 1 for the following reasons: symmetry and minimal disturbance of muscle attachment, reduction of thickness without reduction of width, and reduction in length of the tongue. Use of the V-shaped excision proved most advantageous in the second case where length, rather than thickness, was the only problem. It was hoped that partial glossectomy at an early age might help circumvent the attendant sequelae of macroglossia in patients with Beckwith-Wiedemann syndrome.